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GIORNALE ITALIANO DI DERMATOLOGIA E VENEREOLOGIA

A Journal on Dermatology and Sexually Transmitted Diseases


Official Journal of the Italian Society of Dermatology and Sexually Transmitted Diseases
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Giornale Italiano di Dermatologia e Venereologia 2000 August;135(4):501-4

language: Italian

Multicentric reticulohistiocytosis. Description of a clinical case

Richetta A., Faiola R., Divona L., Calabretta F., Sansolini T., Innocenzi D., Bottoni U., Calvieri S.

Università degli Studi di Roma, «La Sapienza» - Roma Istituto di Clinica Dermatologia Azienda Policlinico Umberto I - Roma


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Multicentric reticulohistiocytosis (MR) is a rare multisystem disease of unknow etiology with a female predominance. It is characterized by nodular-erythematous lesions localized in the skin, mucosa and subcutaneous tissues. It begins with, symmetric, progressive and destructive polyarthritis with predilection for the distal interphalangeal joints of the hands. Muscular, cardiovascular and pulmonary manifestations have occasionally been described. Multicentric reticulohistiocytosis is frequently associated with malignancy like melanoma, carcinoma of the breast, stomach, lung, ovary, colon, pancreas and non-neoplastic pathology like thyroid disorders, diabetes mellitus and tubercolosis. The authors describe the case of a 52 years old woman who presented violet papule-nodular lesions of irregular shape with a maximum diameter of 1 cm, localized on the face, in the oral mucosa and on the left thigh. These lesions occurred about one year before, without any subjective symptom. Histological examination showed the presence of a dermic infiltration by histiocytes and multinucleate giant cells with a ground-glass eosinophilic cytoplasm. Immunohistochemical analysis showed a positive staining for the KP1 (CD68), UCHL1 (CD45) Ki-67, S100 and lysozyme antigens. CD 1A, CD 79 and VIII antigens immunostaining were negative. Radiological examinations documented diffuse polyarthritis. Electron microscopic analysis, evidenced the presence of electron-dense lysosomes and the absence of Birbeck granules. On the basis of clinical, histopathological, immunohistochemical, ultrastructural and instrumental data the diagnosis of multicentric reticulohistiocytosis was made. Therefore the patient underwent a treatment with methotrexate with a remarkable improvement of clinical symptoms.

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